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The spectrum of celiac disease: epidemiology, clinical aspects and treatment

Abstract

Celiac disease is a gluten-sensitive enteropathy that affects people of all ages worldwide. This disease has emerged as a major health-care problem, as advances in diagnostic and screening methods have revealed its global prevalence. Environmental factors such as gluten introduction at childhood, infectious agents and socioeconomic features, as well as the presence of HLA-DQ2 and/or HLA-DQ8 haplotypes or genetic variations in several non-HLA genes contribute to the development of celiac disease. Growing insight into the variable clinical and histopathological presentation features of this disease has opened new perspectives for future research. A strict life-long gluten-free diet is the only safe and efficient available treatment, yet it results in a social burden. Alternative treatment modalities focus on modification of dietary components, enzymatic degradation of gluten, inhibition of intestinal permeability and modulation of the immune response. A small group of patients with celiac disease (2–5%), however, fail to improve clinically and histologically upon elimination of dietary gluten. This complication is referred to as refractory celiac disease, and imposes a serious risk of developing a virtually lethal enteropathy-associated T-cell lymphoma.

Key Points

  • Celiac disease is a gluten-sensitive enteropathy that has emerged as a major health-care problem, as its global prevalence is increasing

  • Advances in diagnostic and screening methods have contributed to the apparent increase in disease prevalence, but evidence also suggests the existence of a real increase caused by environmental changes

  • Diverse environmental, genetic and socioeconomic factors contribute to the development of celiac disease

  • Growing insight into the clinical presentation of celiac disease has resulted in novel diagnostic, prognostic and therapeutic dilemmas

  • Although alternative treatment modalities that reduce the need of dieting are being developed, the only safe and effective therapy available so far is a life-long gluten-free diet

  • 2–5% of patients with celiac disease develop refractory celiac disease, a serious complication that is associated with a 50% risk of developing lymphoma, which has a poor prognosis

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Figure 1: Algorithm for diagnosis of uncomplicated celiac disease.
Figure 2: Algorithm for diagnosis of complicated celiac disease.

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Correspondence to Greetje J. Tack.

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Greetje J. Tack has received an unrestricted grant from AstraZeneca. Chris J. J. Mulder has received financial support from AstraZeneca, DSM Food Specialties, Fujinon, Janssen-Cilag and Nycomed. The other authors declare no competing interests.

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Tack, G., Verbeek, W., Schreurs, M. et al. The spectrum of celiac disease: epidemiology, clinical aspects and treatment. Nat Rev Gastroenterol Hepatol 7, 204–213 (2010). https://doi.org/10.1038/nrgastro.2010.23

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